1. SUPPORT GROUP EVENTS
a. SDS/MSA Support Group Regional Meeting A Huge Success!

SDS/MSA Support Group Regional Meeting
Chicago Hilton
September 13-15, 2002

We had the most wonderful and uplifting meeting we have ever had. The meeting
started off with a social hour on Friday night. 35 people showed up for snacks,
beverages and lots of conversation.

Saturday we opened the meeting day with a breakfast buffet. Don Summers
opened the meeting with greetings and introductions. We counted 80 noses in our
meeting. These noses belonged to Physicians, patients, caregivers, family
members, other professional staff and SDS/MSA Support Group Board members. They
came from CA, CO, OK, MO, MN, IN; WI, OH, GA, PA, KY, TX, FL and of course IL.
We even had a Doctor who was passing by in the hallway who had never heard of
SDS/MSA and asked if she could sit in on the meeting. She and the 79 other noses
were educated by Dr. Janice Gilden, our Host; who spoke on Treatment of
Orthostatic Hypotension; Dr. Tom Chelimsky who spoke on MSA and Movement
Disorders; Dr. David Robertson who spoke on What's New in MSA and Research; Dr.
Fetnat Fouad-Tarazi who spoke on Cardiac Considerations; Dr. Nalinaskha Joshi
who spoke on Sleep Disorders; Barbara Fox M.S.W. a social worker; Lori Hedges
from Horizon Hospice and Randee Sable from Resurrection Home Health.

Saturday afternoon we had a Round table discussion where the audience asked
the doctors questions. We also had breakout sessions for the patients with Don
Summers as the mediator and Lyn Wood had the caregivers and family members. The
meeting ended at 4:30 p.m.

Sunday there were 25 for breakfast and informal meeting. There were lots of
friendships made and information sharing.

A big Thank you to all Doctors, patients, caregivers and family members for
making the meeting a huge success.

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The SDS/MSA Support Group is a Non Profit corporation devoted to reaching and
assisting the Patients, Caregivers, Family Members and Physicians who are
dealing with Shy-Drager Syndrome (Also known as Multiple System Atrophy).

Our mission is to educate and support these people by establishing a
never-ending circle of information among all involved. This has become known as
the " Circle of Hope".

Don Summers, full of energy and love for MSA patients, families and doctors,
welcomed us all and officially opened the SDS/MSA Support Regional Meeting in
Chicago. Eighty people were in attendance with representatives from California
to New York and many states in between, including a large turnout from the
Chicagoland area. Dr. Janice Gilden, our host, gathered together an expert panel
of doctors to discuss Multiple System Atrophy. From the start, she set a tone of
openness about the variety of symptoms and experiences.

Dr. Tom Chelimsky (Case Western Reserve University) presented the first talk.
Billed as a neuroscience talk - 'What is SDS/MSA and Autonomic Dysfunction' - it
turned out to be much more. Dr. Chelimsky explained that while there was unity
in the disease process itself from person to person with MSA, a wide variety of
symptoms are seen in the disease. Observations of MSA patients with common
symptoms have given way to descriptions of three separate types of MSA called
Striatonigral Degeneration, sporadic Olivopontocerebellar Atrophy and Shy-Drager
Syndrome.

In Parkinson's patients, the substantia nigra is the main area of the brain
affected. 80% of dopamine making cells die before patients present with
neurologically noticeable symptoms. On diagnosis, Parkinson's patients are then
given the drug L-dopa. In that disease, the way L-dopa works is somewhat
understood. More (multiple) areas of the brain, I should add, are damaged in MSA
than in Parkinson's Disease, hence the name Multiple System Atrophy. Important
to his talk, a primary area of the brain damaged in MSA is the striatum. There
is as yet no known drug to repair damage to cells in this area. The two areas
mentioned (the striatum and the substantia nigra) do work so closely together
that if one is damaged, the other is damaged as well, as seen on autopsy.

This intimate relationship brings us to the first form of MSA, striatonigral
degeneration (SND). Dr. Chelimsky described this form as many things getting
smaller, i.e. slower and smaller movements, shorter gait, fine tremor,
smaller/softer voice, smaller handwriting. Parkinson's medications may help
somewhat, but patients will not see the same relief that Parkinson's patients
experience. In contrast to SND, the next form of MSA, sporadic
Olivopontocerebellar atrophy (OPCA), is marked by damage to the cerebellum;
movements are, thus, often bigger, i.e. lack of coordination, loss of balance,
irregular handwriting, slurred speech, and changes in volume up and down. In the
last form, Shy-Drager Syndrome, autonomic symptoms predominate including
orthostatic hypotension and perhaps abnormal body sweating, as opposed to the
parkinsonian or cerebellar symptoms. There are a host of other symptoms from
bladder problems to sexual dysfunction which MSA patients often have. And of
course, patients get a smattering of symptoms from two or even all three forms
of MSA - hence the now common Neapolitan Ice Cream analogy. (See footnote for an
explanation of this analogy.) And as you might now surmise, L-dopa does even
less good, if any, in OPCA and Shy-Drager forms of MSA. There is as yet no
equivalent to L-dopa for MSA patients.

Dr. Fetnat Fouad-Terazi (Cleveland Clinic) gave the second talk, 'Cardiac
Considerations'. Simply put, she pointed out the importance of differentiating
the various causes of syncope (fainting) and postural tachycardia (fast heart
rate upon standing). Such careful attention to causes can help determine the
appropriate course of medication as well as when a treatment may be inadvisable.

Dr. Nalinaskha Joshi (Saint Mary of Nazareth Hospital Center) followed with
'Sleep Disorders'. He kindly provided an outline handout and gave a lucid
presentation on these common MSA problems. After discussing the physiology of
sleep disturbances in MSA patients, he went over treatment regimes including
mechanical options (CPAP and BIPAP) and surgical options. Other helpful
reminders were sleep hygiene (no TV, radio and phone in the bedroom), avoidance
of alcohol a few hours before bed, and regular sleep hours.

Next up, Dr. Janice Gilden (Chicago Medical School and Saint Mary Of Nazareth
Hospital Center), a Midodrine expert I should add, presented 'Treatment of
Orthostatic Hypotension'. OH sufferers were proud to hear such eloquent
treatment options and plans available to new patients now having to battle drops
in blood pressure. Two of the most favored medications are Florinef and
Midodrine (marketed as ProAmatine in the United States and Gutron overseas),
although she did review less commonly prescribed treatments which may be more
appropriate in individual cases. Florinef increases blood volume in order to
help prevent drops in blood pressure. Midodrine acts by constricting blood
vessels and, thus, raising the patient's blood pressure. Of the two medications,
Midodrine has proven more beneficial. Doctors, now, often add one of these
medications to the other in order to attain the desired result, adjusting
dosages accordingly. Also to be taken into account, Dr. Gilden noted that
Midodrine is most effective within one hour and may last from two to six hours
depending on the person. As for supplementary therapies, salt remains a
significant volume building agent. Moreover, recent studies have shown water to
be on average the most important and effective agent to raise blood pressure. We
must not forget to make use of this vital tool. Other tricks Dr. Gilden
mentioned were elevating the head of the bed at night, consuming smaller meals
because of resultant drops in blood pressure after large meals, and timing
medicine and water intake with mealtimes in order to minimize drops in blood
pressure that can occur after eating. Lastly, she highly recommended that
patients be seen by autonomic specialists due to their specific knowledge and
experience in the complexities of blood pressure regulation.

Before the final talk of the day, we were greeted by a vibrant and incredible
social worker, Barbara Fox (Saint Mary of Nazareth Hospital Center). She
discussed 'Emotions, Stress, Counseling and Coping', a topic that held
everyone's attention and offered more than we could realize. Once the emotional
door was open, two short but important talks were given by Horizon Hospice and
Resurrection Home Health Care -- services of which we should all be aware as
options.

Before lunch, Dr. David Robertson (Vanderbilt University) shared his
expertise on some research advances. To begin with, some 13 years ago only 4
physicians attended the American Autonomic Society meeting. Last year, eighty
doctors were in attendance. Quite an improvement! Of research note, he presented
initial findings of a recent paper he read which suggests doses of CoQ10 at
1200mg/day could be of benefit in slowing Parkinson's. Then of special mention,
we received a short genetics seminar. The DNA in MSA patients, he said, was of
sound, proper structure. However, there is an improper folding of the
polypeptides throughout the DNA. The protein from this faulty mechanism then
builds up in certain cells forming inclusions known as glial cytoplasmic
inclusions. This genetic finding could be important in advancing the
understanding of MSA. (Note: Dr. Robertson does not use PET scans as a
diagnostic tool for MSA, only as a research tool.)

A delightful lunch was followed by a panel discussion with the aforementioned
specialists. From the topic of support stockings to double vision, the doctors
tried to help. For instance, if you live up North where stockings can also keep
you warm, a man may actually wear them. In cases where patients do wear support
hose, thigh-highs with a tight girdle were suggested for ease of getting on and
off as well as going to the bathroom. On this same topic, be sure check your
prescription in order to purchase stockings with sufficient tightness. And, do
not forget to take the stockings off when lying down; without such diligence,
they will not be effective when the patient returns to a standing position.

As for double vision, it may be caused by orthostatic hypotension if it
occurs only when standing. If it occurs when standing and laying down, then it
may well be a symptom of MSA. And finally let me mention the topic of memory.
Dementia, Dr. Robertson said, is usually not associated with MSA. Indeed, it may
indicate a related disorder called Diffuse Lewy Body Disease. (Note: MSA
patients may have cognitive impairment. Memory remains intact but there is a
retrieval of memory problem. Additionally, executive functioning can be
impaired. These are primarily frontal lobe problems.) Neuropsychologists are
specifically trained to diagnosis cognitive difficulties. As for research
elsewhere in the US and abroad, discussion among the panel was not forthcoming.
Dr. Chelimsky did though, mention that the Multiple System Atrophy Newsletter
prepared by Pam Bower was a good source for current research study updates.

The day was brought to a close by two separate open dialogue patient and
caregiver sessions with Don Summers and Lyn Wood respectively. And, Sunday
afforded a meeting of the minds wherein the business side of things was
discussed.

In conclusion, what does this conference mean for MSA patients?

- The word about orthostatic hypotension medications is getting out.
Midodrine use is becoming more prevalent, often in conjunction with Florinef.
Doctors are becoming more flexible about the timing and dosage of Midodrine
taking into account each patient's individual reaction to the medication as well
as climate, elevations in blood pressure and other factors.

- On the sleep front, polysomnography tests (PSG) are important due to the
prevalence of sleep disorders in MSA. Appropriate prescribed devices and/or
surgery may be helpful in alleviating symptoms of sleep apnea, etc. But also, it
is important to know that we can work on training our brains with good sleep
hygiene so that the disease does not have as great an effect on disturbing our
rest.

- As for promise in research, an understanding of the pharmacology of the
striatum (for those familiar with the term basal ganglia, it is part of the
striatum) could lead to an L-dopa of sorts for MSA. In that case, a medication
could help alleviate many MSA symptoms; but as in Parkinson's disease, it would
not be a cure.

- A bigger possible breakthrough was described on the level of DNA wherein
the prevention or correction of the folding problem could avert the formation of
glial cytoplasmic inclusions in the multiple areas of the brain.

- Where research falls short, we continue to live our lives. Social workers
can be a great help in coping with illness for the patient and the caregiver.
When the patient is homebound, home health services can play a vital role in
maintaining dignity and care. Hospice provides a remarkable service to those who
wish to remain home during the final stages of the disease.

- Laughter, friendships, questions, discussions, and even some answers marked
this conference as another rewarding gathering led by Don Summers.

Thank you Don!

- Zac Carter

______________________________________________________

Footnote: MSA and the Neapolitan Ice Cream Analogy
by Pam Bower

Think of MSA as the 3 flavoured ice cream called Neapolitan which has
vanilla, chocolate and strawberry all mixed together. Imagine if you took one
scoop of that ice cream and put it in a dish, then took another scoop and put it
in another dish and compared the two dishes. You would notice that there is not
the exact same amount of vanilla, chocolate and strawberry in both of the
dishes.

Multiple System Atrophy is the same as the Neapolitan ice cream. There are
three flavours included in MSA:

1. Shy-Drager Syndrome (SDS) - Think of it as the strawberry ice cream
2. Olivopontocerebellar atrophy (OPCA) - Think of it as the vanilla ice cream
3. Striatonigral Degeneration (SND) - Think of it as the chocolate ice cream

Whether someone is told they have SDS or OPCA or SND they all have one scoop
of Neapolitan ice cream in their dish. They all have Multiple System Atrophy.

If their symptoms are mainly orthostatic hypotension or urinary incontinence
they have mostly strawberry ice cream in their dish (SDS). If their symptoms are
mainly cerebellar ataxia they have mostly vanilla ice cream in their dish (OPCA).
If their symptoms are mainly tremors they have mostly chocolate ice cream in
their dish (SND). If they have all of the above symptoms then they might have
nearly equal amounts of chocolate, strawberry and vanilla in their dish.

Recognizing a growing need for therapeutic intervention in MSA, the EMSA-SG
was formed in 1999 by 20 research groups in eleven European countries (Germany,
Austria, France, United Kingdom, Portugal, Spain, Italy, Sweden, Denmark,
Slovenia and Israel). EMSA-SG is coordinated by Werner Poewe and Gregor Wenning
at the University of Innsbruck. In March 2001, EMSA-SG received EC support for a
three- year project with the 5th framework program. The project aims to
establish a European MSA Registry (EMSA-R), a unified MSA rating scale (UMSARS)
as well as a "Core Assessment Program for Interventional Therapy" (CAPIT) in MSA
(CAPIT-MSA). CAPIT-MSA will be designed similar to previous EC sponsored
concerted efforts in Parkinson's disease (CAPIT-PD, Defer 1999) and Huntington's
Disease (CAPIT-HD, Quinn 1996). CAPIT-MSA will comprise a novel set of EMSA-SG
diagnostic criteria, a novel Unified Rating Scale (UMSARS) and additional
investigations including autonomic function and urodynamic tests as well as
structural and functional brain imaging. Task forces have been set up to promote
development of the CAPIT components. The CAPIT-MSA trial protocol will be
designed and validated through the first ever prospective natural history study
of European patients. During the natural history study, EMSA-SG will facilitate
future research into ecogenetics and molecular pathology of MSA by virtue of
decentralized DNA and brain tissue banking led by Thomas Gasser and Andrew Lees.

These activities will hopefully lead to clinical trial activity within the
next few years. A phase II growth hormone intervention trial has already been
launched in four EMSA-SG sites. EMSA-SG has established close ties with the
Northern American MSA Study Group (NAMSA-SG) chaired by Cliff Shults, San Diego,
CA, USA, who are presently waiting for NIH approval of their work program, which
includes a natural history study. Although financial support can only be offered
to official EC partners, EMSA-SG welcomes new affiliates in the Study Group who
will be regularly updated on the work program and upcoming meetings. A homepage
has been set up for all those wishing to contact the Study Group (www.emsa-sg.org/).

The Cleveland Clinic Foundation is beginning a study investigating the
effects of the drug Hytrin (Terazosin) on improving the symptoms commonly
associated with Multiple System Atrophy. Hytrin is a commonly used, FDA-approved
medication used in the treatment of Benign Prostatic Hypertrophy (BPH) (to
relieve urinary retention) and Hypertension (to lower blood pressure). Patients
interested in participating would undergo a neurological and physical evaluation
including questionnaires and timed motor tests. Subjects are then randomized,
like a flip of a coin, to either Hytrin or a placebo for several weeks and
undergo a series of neurological and physical evaluations. Neither you nor your
doctor will know if you are on the study medication. Every one or two weeks for
12 weeks (end of study), subjects will undergo further neurological evaluations.

For more information, contact Ruthie Kolb, at (216) 444-4598 or
Dr.Thyagarajan Subramanian at (216) 444-4270.

We are seeking male and female patients to voluntarily take part in a
clinical research study. Patients must be aged 18 or older and diagnosed with
symptomatic orthostatic hypotension (low blood pressure while in the upright
position) due to Parkinson's disease, multiple system atrophy, pure autonomic
failure or autonomic neuropathies (i.e. neurogenic orthostatic hypotension).
Symptoms of low blood pressure include dizziness, lightheadedness, changes in
vision and generalized weakness upon standing. The main effect of the drug being
studied is to increase blood pressure in the upright position so symptoms will
decrease.

The purpose of this clinical study is to further assess the clinical benefit
of midodrine hydrochloride (ProAmatine ®), an approved treatment for orthostatic
hypotension. During the course of the study, participants will receive either
ProAmatine ® or a placebo. Assessments will be made using questionnaires that
measure symptom and activity levels. Blood pressure in the lying down and
standing positions will be measured at each visit.

You should be aware that because ProAmatine ® can cause marked elevation of
blood pressure while in the lying down position, it should be used in patients
whose lives are considerably impaired despite standard clinical care. The
indication for use of ProAmatine ® in the treatment of symptomatic orthostatic
hypotension is based primarily on a change in a surrogate marker of
effectiveness, that is, an increase in systolic blood pressure measured one
minute after standing, a surrogate marker considered likely to correspond to a
clinical benefit. At present, however, clinical benefits of ProAmatine ®,
principally improved ability to carry out activities of daily living, have not
been verified.

ProAmatine ® is contraindicated in patients with severe organic heart
disease, acute renal disease, urinary retention, pheochromocytoma or
thyrotoxicisis. ProAmatine ® should not be used in patients with persistent and
excessive supine hypertension.

Please consult with your physician to see whether you might benefit from
participation in this study.

The length of this study is approximately 8 weeks with a minimum of 7
required office visits. Additional office visits may be necessary. Individual
patient participation could be longer or shorter depending on the number of site
visits needed. Patients will receive all study- related procedures at no charge
and will be financially compensated for completed site visits.